• Poonam Aggarwal and Pabina Rayamajhi.
• Department of ENT and Head & Neck Surgery, TU Teaching Hospital, Maharajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.
• JNMA J Nepal Med Assoc. 2020 Dec 31; 58 (232): 1080-1082.

AbstractCongenital external canal atresia is one of the congenital ear anomalies that can occur in patients. Similarly, congenital cholesteatoma is also another congenital disease that is often diagnosed in early adulthood. Both the above-mentioned diseases can occur independently but the presence of both these entities is a rare occurrence and needs a high degree of suspicion aided by a computed tomography scan to make the diagnosis. We are presenting a case of a sixteen-year-old patient who presented with unilateral ear anomaly, earache, facial palsy, and postaural swelling and was diagnosed as a right sided congenital aural atresia with congenital cholesteatoma. He was surgically managed with right-sided modified radical mastoidectomy with canaloplasty and closure of mastoid fistula under general anesthesia.

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